Nephrogenic nephroma was individualized by A. Pagès in 1980 as a rare renal neoplasia that mainly occurs in adolescents and adults and presents a benign outcome. The tumor is composed of very small tubular, papillary, and glomeruloid structures. Immunohistochemistry and electron microscopy show that the tumor is a proliferation of primitive epithelial cells that mimic the embryonal nephron. This neoplastic entity has often been confused with a nephroblastoma, although it is a benign tumor that is cured by simple resection. Further studies are needed to define its exact incidence and its histogenesis.